Lhermitte - Duclos Disease with Syrinx: Case Report and Literature Review

Khursheed NAYIL; Maqbool WANI; Altaf RAMZAN; Feroze SHAHEEN; Iqbal LONE; Abrar WANI

doi:10.5137/1019-5149.JTN .2989-10.1

Issue release date: 12.11.2024

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Turkish Neurosurgery 2011 , Vol 21 , Num 4

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Lhermitte - Duclos Disease with Syrinx: Case Report and Literature Review

Khursheed NAYIL, Maqbool WANI, Altaf RAMZAN, Feroze SHAHEEN, Iqbal LONE, Abrar WANI

SKIMS, Department of Neurosurgery, Kashmir, India DOI : 10.5137/1019-5149.JTN .2989-10.1 BACKGROUND: Lhermitte-Duclos disease is typified by a hamartomatous lesion of the cerebellum. It is usually seen in females. The usual presentation is of raised intracranial pressure along with cerebellar signs.

CASE REPORT: We report an 18-year-old female patient who presented to us with history of headache and gait ataxia and was observed to have Lhermitte-Duclos disease with cervical cord syrinx on imaging.

DISCUSSION: To our knowledge this is the fifth case of Lhermitte-Duclos disease with syringomyelia in the pediatric age group. The treatment of this condition is decompression of the lesion. Malignant transformation never occurs and the prognosis is excellent. Keywords : Lhermitte-Duclos disease, T-2 shine through phenomenon, Tigroid appearance, Cerebellar tumors, Gangliocytoma, Hydrocephalus, Syringomyelia

Corresponding author : Altaf Ramzan, drauramzan@yahoo.com